It will be necessary to consider these apart from accessory and inconstant symptoms, by the presence of which they are often masked.
The essential symptoms are:
(A) Mental deterioration;
(B) Disorders of motility;
(C) Pupillary disturbances;
(D) Changes in general nutrition.
It presents two fundamental characteristics:
(1) It affects all the psychic functions in their ensemble;
Let us analyze rapidly the elements constituting this mental deterioration.
It is profoundly affected from the beginning. The amnesia is both anterograde, by default of fixation, and retrograde, by destruction of impressions. It is essentially incurable.
The disappearance of old impressions probably follows the law of amnesia; but its course is so rapid that it is difficult to demonstrate this fact. The impressions of youth and childhood become very rapidly effaced, so that after a relatively short period only a few confused and distorted recollections remain in the mind of the patient, and these are only with great difficulty recovered from the general wreck.
Their disorders are manifested by:
(I) More or less complete loss of orientation in all its forms;
(II) More or less confused perception of the external world.
The clouding of consciousness and the confusion attain in the terminal period, and in certain forms in the beginning, an extreme intensity.
The attention of the patient is difficult to rouse as well as to fix. In some cases early in the disease, in phases of excitement, there may be flight of ideas. This, however, is of exceptional occurrence; as a rule there is sluggish formation of associations of ideas demonstrable by psychometry or by an ordinary clinical examination. In the cases in which some mental activity is still possible there is rapid mental fatigability, so that the patient is no longer able to do mental work of any complexity; in advanced stages even the simplest intellectual operations are impossible.
Its changes are characterized by morbid indifference and irritability, associated in the manner already described.1 Both the indifference and the irritability are apt to be very marked. The general paralytic takes no interest in his own business or in the welfare of his relatives.
1 See Part I, Chapter IV (Dementia Praecox).
Grave occurrences fail to impress him. On the other hand, he is subject to fits of terrible anger on the slightest provocation.
The moral sense and regard for conventionalities disappear entirely. The patient commits the most ridiculous and most revolting acts with perfect serenity and is astonished when his liberty of action is interfered with.
Its disorder finds expression in the patient's total lack of insight into his condition. Together with the amnesia, it explains the inconsistencies in the patient's conduct and speech; he is unable to appreciate the most flagrant contradictions. To a given question he gives the first answer that enters his mind, whether it happens to be false or correct, absurd or plausible.
(/) Reactions. - As might be expected, they are always impulsive. The reflections, that is to say the series of associations preceding the act, become more and more reduced. As the patient sees what he wants he immediately takes it. He wants an object that he sees exposed for sale in a shop - he takes it and carries it off without taking the trouble to pay for it. A paralytic leaning over the parapet of a bridge drops his cane. To recover it, reasoning that a straight line is the shortest distance between two points, he jumps after it into the water. Stereotyped movements (movements of sucking, grinding the teeth, etc.) and negativism are frequent. Cataleptoid attitudes are occasionally seen.
The fundamental motor disturbances, the only ones that need occupy us here, are three in number: (a) Progressive muscular weakness; (6) Tremors; (c) Motor incoordination.
It is most marked in the latter periods of the affection, when it accompanies the general cachexia. It involves all the muscles and is associated with more or less pronounced atrophy so that there is more or less complete disability.
Unlike the muscular weakness, these constitute an early symptom. They are of two kinds: fibrillary tremors and tremors en masse.
(I) The fibrillary tremors consist in rapidly repeated contractions of very small groups of muscular fibers. It is a sort of twitching. It is observed chiefly in the tongue and in the peri-buccal muscles.
(II) Tremors en masse usually appear as coarse oscillations, irregular in frequency and in amplitude. They become evident on voluntary movements and form a sort of point of transition between true tremors and muscular ataxia. They are seen especially in the upper extremities and in the tongue. The tongue projected from the mouth executes to-and-fro movements very aptly described by Magnan as "trombone movements."
This first becomes evident in the most delicate movements and manifests itself early by impairment of speech and handwriting.
I. The impairment of speech, clearly apparent in advanced stages, is sometimes difficult to notice at the beginning and becomes evident only on resorting to special tests, such as prolonged reading in a loud voice or the pronunciation of test-phrases: Methodist Episcopal, fourth cavalry brigade, national intelligence, etc.
Sometimes the impairment of speech becomes less evident or even disappears temporarily during excitement. Often it becomes accentuated after apoplectiform or epileptiform attacks.
It is of various types, the principal of which are the following:
(a) Drawling, tremulous, indistinct speech;
(8) Scanning speech analogous to that of disseminated sclerosis;
(7) Hesitating speech: the patient stops in the middle of a word and seems to hesitate before finishing it;
(5) Omission of one or more syllables: the patient pronounces, for instance, "Methist Pispal" instead of Methodist Episcopal;
(e) Reduplication of one or of several syllables, as "constititutional";
(f) Interchange of syllables: "constutitional."
These types may be combined so as to form mixed types of infinite variety.
II. The handwriting is characterized by its irregular appearance, and by the coarse tremors seen in the strokes. These motor disorders are always associated with phenomena of intellectual origin: omissions, or, on the contrary, repetitions of letters, syllables, or words, numerous glaring orthographical errors. All these features impart to the handwriting of general paralysis its characteristic aspect.
Usually the patient is totally unconscious of these symptoms. If accidentally he notices them, he is neither surprised nor alarmed. The explanations which he gives are childish: he does not speak well because he has lost a tooth, or he writes with difficulty because his hands are cold.
Slight in the beginning, the impediment of speech and the impairment of handwriting become progressively aggravated, so that in the terminal stage of the disease the writing becomes shapeless scribbling and the speech unintelligible stammering.
At the end of the disease it is almost constant to note disturbance of deglutition caused by paresis and incoordination of the pharyngeal muscles, which may entail death by suffocation.
These appear sometimes very early.
They are dependent upon an internal ophthalmoplegia of gradual and progressive development (Baillet and Bloch), which is manifested by changes in the shape, size, and reactions of the pupil.
The pupil loses its circular shape and becomes oval or irregular. This symptom seems to be frequent, but of its diagnostic value little is known.
1 Mignot. Contribution a I 'etude des troubles pupillaires dans quel-gues maladies mentales. These de Paris, 1900.
These are of three kinds:
I. Myosis, at times so marked that the pupils are reduced to pin-hole size.
II. Mydriasis, also very well marked in some cases.
III. Inequality, which may be produced by three different mechanisms:
(α) One pupil is normal, the other myotic or mydriatic;
(β) One pupil is mydriatic, the other myotic;
(γ) Both pupils are mydriatic or myotic, but are unequally dilated or contracted.
It is important, in order to make a satisfactory examination of the pupils, to place the patient in such a light that both eyes receive an equal amount of illumination. It is also important to vary the intensity of illumination, because an inequality that appears doubtful in a strong light may become very evident in a weaker light, and vice versa.
Pupillary inequality is sometimes congenital. Moreover, it is encountered in many affections other than general paralysis: dementia praecox, compression of the sympathetic nerve, etc.; therefore, it does not by any means constitute a pathognomonic sign.
These consist in changes in the light reflex, or the accommodation reflex, or both. They are either binocular or monocular.
Disorders of the pupillary reactions may be associated as in the Argyll-Robertson type: abolition of the light reflex with persistence of the accommodation reflex. This combination is, however, considerably less frequent in general paralysis than in tabes.
At the beginning of the disease the reactions are not completely abolished, but are simply paretic.
It is not uncommon for the speech defect and the pupillary signs to persist through complete mental remissions.
Though constant and very important they have thus far received but little attention. Clinically we find changes in weight and in the urinary secretion.
The onset is almost always marked by considerable loss of weight. Later the weight varies with the clinical form.
In the excited and depressed forms of rapid evolution the loss of weight is marked and progressive, and the patient rapidly becomes cachectic.
In the expansive or demented forms the weight often rises after the initial fall, the patient then becoming corpulent and remaining so until the terminal stage, when the weight may fall suddenly and continue to drop as marasmus is established.
Organic crises may be noted in the course of the disease (Arnaud); they consist in transitory but considerable loss of weight, the cause of which is unknown.
The changes in the urinary secretion indicate general sluggishness of nutrition. They have been especially studied in connection with the second period of the disease. The principal ones are polyuria, low specific gravity of the urine, slight albuminuria, very considerable diminution of urea and of phosphates, and increase of chlorides.1
A study of the blood changes might also be of great interest. The work already done along this line is unfortunately very scant and inconclusive. Capps 2 found a slight diminution of haemoglobin and of the red blood cells.